Families Affected by Huntington’s Disease Report Difficulties in Communication, Emotional Involvement, and Problem Solving

Jona, Celine M. H.; Labuschagne, Izelle; Mercieca, Emily-Clare; Fisher, Fiona; Gluyas, Cathy; Stout, Julie C.; Andrews, Sophie C.

doi:10.3233/jhd-170250

Cited by 17 publications

(7 citation statements)

References 21 publications

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“…Family functioning is the function of the family system itself, which refers to the ability of the family to function effectively to meet basic needs and manage conflicts ( Jona et al, 2017 ). The circumplex model of marital and family systems considers family as functioning in three dimensions: family cohesion, flexibility (initially called adaptability), and communication ( Olson, 2000 ).…”

Section: Introductionmentioning

confidence: 99%

Family functioning and adolescent depression: A moderated mediation model of self-esteem and peer relationships

Huang

Yao

et al. 2022

Front. Psychol.

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In consideration of family system theory, the vulnerability model of depression, and the stress buffering model of social support, the current study examined the effect of family functioning on adolescent depression, the mediating effect of self-esteem, and the moderating effect of peer relationships. A sample of Chinese adolescents (n = 562, 47.15% male, 52.85% female, mean age 14.33 years, SD = 1.81 years) completed questionnaires regarding family functioning, depression, self-esteem, and peer relationships. The results showed that: (1) family functioning had a significant negative predictive effect on adolescent depression; (2) self-esteem plays a mediating role between family functioning and adolescent depression; and (3) peer relationships have a moderating effect on the relationship between self-esteem and adolescent depression, supporting the moderated mediation model. These results reveal the influence mechanism of family functioning on adolescent depression and have implications for adolescent depression intervention.

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Section: Introductionmentioning

confidence: 99%

Family functioning and adolescent depression: A moderated mediation model of self-esteem and peer relationships

Huang

Yao

et al. 2022

Front. Psychol.

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“…Manifold approaches have been created to assess the role of caregivers and families, to find appropriate measurement and assessment methods, to evaluate quality of life and iden-Brain Sci. 2021, 11, 1621 2 of 13 tify determinants to assess the influence of HD on patients and caregiver burdens [7][8][9][10][11]. Because findings are mostly based on case reports with qualitative interviews, further understanding and validation in a large real-world cohort is necessary.…”

Section: Introductionmentioning

confidence: 99%

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

Achenbach

Saft

2021

Brain Sciences

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Background: In addition to the effects on patients suffering from motor-manifest Huntington’s disease (HD), this fatal disease is devasting to people who are at risk, premanifest mutation-carriers, and especially to whole families. There is a huge burden on people in the environment of affected HD patients, and a need for further research to identify at-risk caregivers. The aim of our research was to investigate a large cohort of family members, in comparison with genotype negative and premanifest HD in order to evaluate particular cohorts more closely. Methods: We used the ENROLL-HD global registry study to compare motoric, cognitive, functional, and psychiatric manifestation in family members, premanifest HD, and genotype negative participant as controls. Cross-sectional data were analyzed using ANCOVA-analyses in IBM SPSS Statistics V.28. Results: Of N = 21,116 participants from the global registry study, n = 5174 participants had a premanifest motor-phenotype, n = 2358 were identified as family controls, and n = 2640 with a negative HD genotype. Analysis of variance revealed more motoric, cognitive, and psychiatric impairments in premanifest HD (all p < 0.001). Self-reported psychiatric assessments revealed a significantly higher score for depression in family controls (p < 0.001) when compared to genotype negative (p < 0.001) and premanifest HD patients (p < 0.05). Family controls had significantly less cognitive capacities within the cognitive test battery when compared to genotype negative participants. Conclusions: Within the largest cohort of HD patients and families, several impairments of motoric, functional, cognitive, and psychiatric components can be confirmed in a large cohort of premanifest HD, potentially due to prodromal HD pathology. HD family controls suffered from higher self-reported depression and less cognitive capacities, which were potentially due to loaded or stressful situations. This research aims to sensitize investigators to be aware of caregiver burdens caused by HD and encourage support with socio-medical care and targeted psychological interventions. In particular, further surveys and variables are necessary in order to implement them within the database so as to identify at-risk caregivers.

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“…It is perhaps surprising, considering MJD is an autosomal-dominant disease, that no studies discussed the inclusion of family members as study participants. The devastating impact families face with autosomal-dominant neurodegenerative diseases is well known 86–88. While family support, peer socialisation and support through physical activity is a facilitator for engagement in physical activity for people with neurodegenerative diseases,89 no studies in this review discussed these factors.…”

Section: Discussionmentioning

confidence: 98%

What is the best way to keep walking and moving around for individuals with Machado-Joseph disease? A scoping review through the lens of Aboriginal families with Machado-Joseph disease in the Top End of Australia

Carr

Lalara²,

Lalara³

et al. 2019

BMJ Open

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ObjectivesMachado-Joseph disease (MJD) is the most common spinocerebellar ataxia worldwide. Prevalence is highest in affected remote Aboriginal communities of the Top End of Australia. Aboriginal families with MJD from Groote Eylandt believe ‘staying strong on the inside and outside’ works best to keep them walking and moving around, in accordance with six key domains that form the ‘Staying Strong’ Framework. The aim of this current study was to review the literature to: (1) map the range of interventions/strategies that have been explored to promote walking and moving around (functional mobility) for individuals with MJD and; (2) align these interventions to the ‘Staying Strong’ Framework described by Aboriginal families with MJD.DesignScoping review.Data sourcesSearches were conducted in July 2018 in MEDLINE, EMBASE, CINAHL, PsychINFO and Cochrane Databases.Eligibility criteria for selecting studiesPeer-reviewed studies that (1) included adolescents/adults with MJD, (2) explored the effects of any intervention on mobility and (3) included a measure of mobility, function and/or ataxia were included in the review.ResultsThirty studies were included. Few studies involved participants with MJD alone (12/30). Most studies explored interventions that aligned with two ‘Staying Strong’ Framework domains, ‘exercising your body’ (n=13) and ‘searching for good medicine’ (n=17). Few studies aligned with the domains having ‘something important to do’ (n=2) or ‘keeping yourself happy’ (n=2). No studies aligned with the domains ‘going country’ or ‘families helping each other’.ConclusionsEvidence for interventions to promote mobility that align with the ‘Staying Strong’ Framework were focused on staying strong on the outside (physically) with little reflection on staying strong on the inside (emotionally, mentally and spiritually). Findings suggest future research is required to investigate the benefits of lifestyle activity programmes that address both physical and psychosocial well-being for families with MJD.

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Families Affected by Huntington’s Disease Report Difficulties in Communication, Emotional Involvement, and Problem Solving

Cited by 17 publications

References 21 publications

Family functioning and adolescent depression: A moderated mediation model of self-esteem and peer relationships

Family functioning and adolescent depression: A moderated mediation model of self-esteem and peer relationships

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

What is the best way to keep walking and moving around for individuals with Machado-Joseph disease? A scoping review through the lens of Aboriginal families with Machado-Joseph disease in the Top End of Australia

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